1 Departments of Pediatrics and Neurology, Bowman Gray School of Medicine, Winston-Salem, NC 27103
2 Department of Human Genetics, Medical College of Virginia, Richmond, VA 23298
To the Editor.
We report a case of sudden death in a patient with presumed biotinidase deficiency and emphasize the importance of suspecting this diagnosis in patients who present with compatible clinical findings.
A nonconsanguineous couple was seen for genetic counseling following the death of their first child, a boy, at 23 months of age. A review of the history revealed that the child was normal until 5
months of age when seizures developed which were eventually controlled with carbamazepine.